Herpes Zoster in Childhood

Herpes zoster is caused by reactivation of latent varicella-zoster virus that resides in a dorsal root ganglion. Herpes zoster can develop at any time after a primary infection or varicella vaccination. The incidence among children is approximately 110 per 100,000 person-years. Clinically, herpes zoster is characterized by a painful, unilateral vesicular eruption in a restricted dermatomal distribution. In young children, herpes zoster has a predilection for areas supplied by the cervical and sacral dermatomes. Herpes zoster tends to be milder in children than that in adults. Also, vaccine-associated herpes zoster is milder than herpes zoster after wild-type varicella. The diagnosis of herpes zoster is mainly made clinically, based on a distinct clinical appearance. The most common complications are secondary bacterial infection, depigmentation, and scarring. Chickenpox may develop in susceptible individuals exposed to herpes zoster. Oral acyclovir should be considered for uncomplicated herpes zoster in immunocompetent children. Intravenous acyclovir is the treatment of choice for immunocompromised children who are at risk for disseminated disease. The medication should be administered ideally within 72 hours of rash onset.

T-lymphoblastic lymphoma with cutaneous involvement

To study dermatological manifestation of T-lymphoblastic lymphoma and to help clinicians in the diagnosis, we report here the case of a 75-year-old patient who presented with violaceous nodules acquired during the last 4 wk and affecting the scalp and right arm. The diagnosis of systemic lymphoma was suggested upon the appearance of cutaneous tumors, palpable lymph nodes and general symptoms including asthenia and weight-loss. The pathology features: positive immunostaining for CD3 and terminal deoxynucleotidyl transferase(Td T) and staging, led us to the final diagnosis of T-lymphoblastic lymphoma(T-LBL) with cutaneous involvement. He received a CHOP regimen as first-line treatment. Unfortunately, the patient relapsed and died 8 mo after the treatment initiation. T-LBL may be diagnosed by skin lesions. Additional immunostaining including Td T and experienced histopathologists are needed to correctly classify this aggressive disease and discuss the correct management including bone-marrow transplantation where appropriate.

阴囊多发性结节的组织病理学发现和手术治疗:5例病例研究

Background:Multiple nodules of the scrotum are uncommonly reported.Their origin is controversial.Treatment is always surgical but the best procedure is still to be determined.Materials and methods:Five new cases are reported with description of the histopathological findings and surgical procedure.Results:Nodules of the scrotum were more frequent in patients with dark skin suggesting an ethnic susceptibility.No other predisposing factors were noted.Screening for disturbances of phosphate or calcium balance was negative.The following histopathological findings were observed:non-calcified epidermoid cysts(3 patients),calcified epidermoid cysts(1 patient)and nodularcalcifications without epithelial or glandular structures(1 patient).Subtotal excisions of the scrotum wall using tumescent anaesthesia were performed in all patients without any significant complications.Cosmetic results were excellent.No new lesionswere observed during the 1-year follow-up period.Conclusions:Most cases of multiple nodules of the scrotum are due to non-calcified epidermoid cysts.The term scrotal calcinosis is therefore probably abusively used bymany authors.Some cases of nodular calcifications may be due to dystrophic calcification of epidermoid cysts,but calcifications may also occur without any visible epithelial or glandular structure.Subtotal excision of the scrotum wall is a safe and effective surgical procedure to treat multiple nodules of the scrotum.Cosmetic results are excellent and recurrences are rare.

英夫利昔单抗诱导和维持治疗中重度银屑病的Ⅲ期临床多中心双盲对照试验

Background: Tumour necrosis factor α(TNFα) is thought to play a part in the pathogenesis of psoriasis. We assessed the efficacy and safety of continuous treatment with infliximab, a monoclonal antibody that binds to and neutralises the activity of TNFα, in patients with psoriasis. Methods: In this phase III, multi centre, double-blind Rial , 378 patients with moderate-to-severe plaque psori asis were allocated in a 4:1 ratio to receive infusions of either infliximab 5 m g/kg or placebo at weeks 0, 2, and 6, then every 8 weeks to week 46. At week 24, placebo treated patients crossed over to infliximab treatment. Skin and nail signs of psoriasis were assessed using the psoriasis area and severity index (PASI ) and nail psoriasis severity index (NAPSI), respectively. The primary endpoint, analysed on an intention-to-treat-basis, was the proportion of patients achi eving at least a 75%improvement in PASI from baseline to week 10. Findings: At week 10, 80%(242/301) of patients treated with infliximab achieved at least a 7 5%improvement from their baseline PASI (PASI 75) and 57%(172/301) achieved at least a 90%improvement (PASI 90), compared with 3%and 1%in the placebo group, respectively (p< 00001). At week 24, PASI 75 (82%for infliximab vs 4%for pl acebo) and PASI 90 (58%vs 1%) were maintained (p< 00001). At week 50, 61%ac hieved PASI 75 and 45%achieved PASI 90 in the infliximab group. Infliximab was generally well tolerated in most patients. Interpretation: Infliximab is effecti ve in both an induction and maintenance regimen for the treatment of moderate-t o-severe psoriasis, with ahigh percentage of patients achieving sustained PASI 75 and PASI 90 improvement through 1 year.

职业继发的多发性黏液样囊肿

We report the case of a 50-year-old woman who presented with eight digital myxoid cysts (DMCs) involving the fingers of both hands. They developed within 12 months of the patient starting a job that involved pushing a garment into an embroidery mould, thus exerting a downward force on the finger tips. The pressure exerted from this force could have potentially damaged the joint synovial capsule, leading to rupture and loss of synovial gel, thus inducing myxoid cysts. This case suggests that DMCs may be related to ation, and to our knowledge, this is only the second reported case of occupationally induced DMCs.

银屑病患者下丘脑-垂体-肾上腺轴对心理应激的反应

Background: Psoriasis may, in some patients, be triggered and/or exacerbated by stress. Objectives: As activation of the hypothalamic-pituitary- adrenal (HPA) axis is critical to a successful stress response we investigated this in patients with psoriasis. Methods: Forty patients with chronic plaque psoriasis and 40 age-matched normal controls experienced three randomly presented acute psychological stressors (cognitive, emotional and social). Serial serum cortisol, pulse rate and blood pressure assessments were undertaken at baseline and following each of the stressors. Salivary cortisol samples were collected at 09.00 h on the day of testing. Results: In control subjects there was a significant (r = 0.38; P < 0.05) correlation between pulse rate and serum cortisol level following the social performance stressor; this was not evident in the psoriasis group (r = 0.07; not significant). Patients who believed that their psoriasis was highly stress responsive had significantly lower salivary cortisol levels at baseline (P < 0.01) and lower serum cortisol levels following the social performance Stressor (P = 0.016) than patients with nonstress-responsive disease who believed that stress had no impact. In contrast, there was no difference between the groups for change in pulse rate poststressor. Conclusions: This study shows that patients with psoriasis, and in particular those whose disease appears to be stress responsive, exhibit an altered HPA response to acute social stress. The implication is that such patients may perhaps be primed to flares of their psoriasis. Whether this is genetically predetermined and/or a consequence of the distress of living with psoriasis remains to be determined.

发生于英国的漆树科皮炎

Background: We present two cases of Toxicodendron dermatitis, one acquired in the United States but presenting in the United Kingdom (UK), the other a recurrent dermatitis following importation of the plant to the UK. Poison ivy, poison oak and poison sumac are native to North America and belong to the genus Toxicodendron. This group of plants is of interest to the dermatologist because they contain a mixture of potent sensitisers which cause a severe allergic contact dermatitis. Conclusions: The dermatitis can present to the dermatologist in Europe after an individual has been in contact with the plant whilst visiting an endemic area. The plants have the potential to grow in Europe and it is therefore possible for an individual to be sensitised and subsequently to develop the rash without leaving the continent.

成功治疗银屑病可改善银屑病患者的特异心理因素,但对患者的一般心理因素无显著改善

Background:Psoriasis has a detrimental effect on patients’quality of life. H owever, there is a relative dearth of information on which aspects of a patient ’s well-being are affected by successful treatment. Objectives:To investigate whether, and to what extent, improvement in the clinical severity of psoriasis induced by photochemotherapy with psoralen plus ultraviolet A (PUVA) translates into meaningful changes in beliefs about psoriasis, coping, stress, distress or disability. Methods:In a prospective study, 72 patients were assessed before PU VA therapy and again when they had achieved clearance of their psoriasis. Result s:Patients demonstrated significant reductions in psoriasis-related disability , psoriasis-related stress or daily hassles and in the frequency of psoriasis- related symptoms. By comparison, there were no significant differences in levels of anxiety, depression or worrying. Similarly, patients’perceptions about cure , potential chronicity, causes, consequences and coping also remained unchanged. Conclusions:These results suggest that while clearance of psoriasis produces a significant reduction in factors specific to psoriasis (disability and stress), it does not impact upon psychological distress, on patients’beliefs about psor iasis or on coping. This observation highlights the complex features of patients ’psychological experience of psoriasis and may provide further impetus for inte gration of psychological interventions into standard care protocols.

延胡索酸酯治疗重症银屑病的58例病例回顾

Background: Fumaric acid esters (FAE) have been used to treat severe psoriasis in northern Europe for over 20 years. A recent systematic review has shown FAE to be an effective systemic treatment for severe psoriasis. However, FAE remain unlicensed in the U.K. Objectives: To present data relating to the efficacy and tolerability of FAE in severe psoriasis and report our experiences of FAE therapy at one U.K. centre. Methods: Patients who had received FAE for severe psoriasis at one U.K. regional referral centre between June 1999 and October 2003 were identified from pharmacy records. Their records were analysed retrospectively. Results: Fifty- eight patients (25 women, 33 men) were identified. Fifty- five (95% ) of the 58 patients had previously used other systemic antipsoriatic therapies with over 70% previously using two or more agents. Thirty- two patients (55% ) showed improvement in their psoriasis with 10 (17% ) being rated as ‘ clear’ or ‘ virtually clear’ by the attending physician. No improvement was seen in 28% patients and 16% showed worsening of their disease. Adverse events were common and were reported in 66% patients. These mainly consisted of abdominal pain (61% ), diarrhoea (55% ), flushing (45% ), nausea (21% ) and malaise (15% ). They led to discontinuation of treatment in 15 patients after a mean period of 4- 7 months. Lymphocytopenia developed during treatment in 57% of patients, all of whom had had a baseline value within the normal range. In only one patient was this considered severe enough to warrant withdrawal of treatment. Conclusions: Our study has shown that FAE are an effective therapy in selected patients with severe psoriasis, even in those who have previously been intolerant of systemic therapy or where it has failed.

局部外用甲基氨基酮戊酸(Metvix~)-PDT治疗颜面和头皮光化性角化症:两种给药法的随机多中心比较研究

Photodynamic therapy (PDT) with topical methyl aminolevulinate (MAL) administered in two treatment sessions separated by 1 week is an effective treatment for actinic keratoses. This open prospective study compared the efficacy and safety of MAL- PDT given as a single treatment with two treatments of MAL- PDT 1 week apart. Two hundred and eleven patients with 413 thin to moderately thick actinic keratoses were randomized to either a single treatment with PDT using topical MAL (regimen I; n=105) or two treatments 1 week apart (regimen II; n=106). Each treatment involved surface debridement, application of Metvix(r) cream (160 mg/g) for 3 h, followed by illumination with red light using a light- emitting diode system (peak wave length 634± 3 nm, light dose 37 J/cm2). Thirtyseven lesions (19% ) with a non- complete response 3 months after a single treatment were re- treated. All patients were followed up 3 months after the last treatment. A total of 400 lesions, 198 initially treated once and 202 treated twice, were evaluable. Complete response rate for thin lesions after a single treatment was 93% (95% CI=87- 97% ), which was similar to 89% (82- 96% ) after repeated treatment. Response rates were lower after single treatment of thicker lesions (70% (60- 78% )- vs 84% (77- 91% )), but improved after repeated treatment (88% (82- 94% )). The conclusion of this study is that single treatment with topical MAL- PDT is effective for thin actinic keratosis lesions; however, repeated treatment is recommended for thicker or non- responding lesions.

外用5-氟尿嘧啶联合冷冻治疗寻常疣无特别益处:一项单中心、双盲、随机、安慰剂对照试验

The role of topical 5-fluorouracil (5-FU) in treating common warts is not well defined. We tried to evaluate the efficacy and adverse effects of combination cryotherapy and topical 5% 5-FU ointment in the treatment of common warts. The study was a single-centre, double-blind randomized placebo-controlled trial. In the study, 80 patients with common warts were randomized into two groups and underwent two 10-second freeze/thaw cycles of cryotherapy with liquid nitrogen once every three weeks for a maximum of five treatments. Between treatments, patients applied either topical 5% 5-FU ointment (group A) or placebo aqueous cream (group B) twice daily. The mean ± SD reduction in wart area was 58.57 ± 0.06% in group A and 65.29 ± 0.06% in group B. In total, 19 patients in group A and 24 patients in group B had wart size reduced by 75% or more (P = 0.50), while 12 patients in group A and 17 patients in group B had clearance of their warts (P = 0.245). Logistic regression with age, sex, smoking status, immune status, site, duration and number of warty lesions, history of previous treatment, and treatment group found that only a history of previous treatment and acral lesions were significant adverse predictors of improvement. There was no significant difference in the number of adverse events between the two groups, although there was a trend towards more pain and blistering associated with topical 5-FU. We concluded that topical 5-FU has no added benefit in treating common warts with cryotherapy.

隐性营养不良性大疱性表皮松解症患者体内的维生素和微量金属元素水平

Background. In recessive dystrophic epidermolysis bullosa (RDEB), a good nutr itional balance is necessary to obtain healing of the chronic wounds. However, i nvolvement of the oral mucosa and oesophagus stenosis may be responsible for sev ere nutritional deficiencies. Objective. In order to propose an adapted nutritio nal management, we studied the vitamin and trace metal status of 14 RDEB patient s. Methods. Height and weight were measured. Plasma levels of albumin, iron, fer ritin, calcium, parathyroid hormone (PTH), folates, vitamins C, D, B12, A, E, B1 , B6, PP and B2, zinc, selenium, carnitine and copperweremeasured. Results. Most patients had a significant growth retardation. We found iron, vitamin D, C, B6, PP, zinc and selenium deficiencies in 36- 70% of the patients, without clini cal expression, except in one case. Vitamin B1, 12, B2, A/RBP, E/lipids and carn itine were normal. The three patients with gastrostomy feeding had better growth but still a protein deficiency and sometimes vitamin C, B6, PP, zinc and carnit ine deficiencies. Conclusion. Vitamin and trace metal deficiencies are frequent in RDEB, even in patients receiving gastrostomy feeding, and often go unrecogniz ed. Regular nutritional evaluation is necessary. Dietary advice and supplements should be given. Enteral feeding by gastrostomy should be discussed in early chi ldhood.

局限性纵向红甲:诊断意义及生理解释

Background: Longitudinal erythronychia (LE) is a term for red streaks in the nail. We describe the range of diseases manifested by localized (single or bifid) LE and explain the underlying physical changes. Observations: Longitudinal erythronychia can be multiple or localized. Multiple lesions typically indicate an inflammatory disease such as lichen planus. When localized, they may be a single or bifid streak arising through a benign or malignant neoplasm, scarring of the dermis or epidermis, or the first stage of an inflammatory process that may evolve into multiple LE. Excision of a localized LE may provide a diagnosis and cure. Incisionalmatrix biopsy ofmultiple LE may provide a diagnosis. Clinical manifestation of LE arises through reduced compression of the nail bed due to loss of bulk of the nail plate with a groove on the undersurface. A streak of thinned nail then allows an enhanced view of a corresponding streak of engorged nail bed. The reduction in nail thickness renders it more fragile with a tendency to split distally. Conclusions: Understanding LE can assist in diagnosis and explanation to the patient. Localized LE may represent a focal tumor or dysplastic process.

先天性鱼鳞病患者25-羟基维生素D显著缺乏的证据

Background: Vitamin D is essential for bone mineralization, and its deficiency may be the cause of skeletal fractures and osteomalacia. Geographical or ethnic factors may modulate the cutaneous synthesis of vitamin D. We hypothesized that major changes in keratinization may similarly alter the cutaneous synthesis of vitamin D. Objectives: To explore calciotrophic hormones, parameters of bone remodelling and bone mineral density (BMD) in nine patients with non-bullous congenital ichthyosis. Patients and methods: Six patients were European, three were North African. Four had received acitretin over a long period of time. A complete biological investigation, including serum and urinary calcium and phosphorus, calciotrophic hormones [intact parathyroid hormone (iPTH), 25-hydroxyvitamin D (25-(OH)D) and 1,25-dihydrox- yvitamin D (1,25-(OH) 2 D)], bone formation and resorption markers, was performed on all patients during the winter season and repeated among four patients after summer. BMD was measured in all patients. Results: All patients had a marked 25-(OH)D deficiency, clearly below the deficiency threshold of 25 nmol/L. Patients from North Africa had a greater deficiency than European patients, perhaps because of the difference in skin pigmentation. iPTH remained normal in European patients but was elevated among the North Africans. After sun exposure, an improvement in vitamin status was visible in only one patient. Bone formation and resorption markers remained normal. Femoral neck osteodensitometry indicated values near the osteopaenic threshold in two young North African females. No deleterious effect of retinoids on vitamin D metabolism was observed. Conclusion: Patients, and in particular pigmented patients, with congenital ichthyosis present a severe deficiency in vitamin D. Care provided to protect the skeletal future of these patients involves measuring BMD and prescribing supplementation.

Staphylococcal-scalded skin syndrome:evaluation,diagnosis,and management

Background Staphylococcal-scalded skin syndrome(SSSS),also known as Ritter disease,is a potentially life-threatening disorder and a pediatric emergency.Early diagnosis and treatment is imperative to reduce the morbidity and mortality of this condition.The purpose of this article is to familiarize physicians with the evaluation,diagnosis,and treatment of SSSS.Data sources A PubMed search was completed in Clinical Queries using the key terms"Staphylococcal scalded skin syn-drome"and"Ritter disease".Results SSSS is caused by toxigenic strains of Staphylococcus aureus.Hydrolysis of the amino-terminal extracellular domain of desmoglein 1 by staphylococcal exfoliative toxins results in disruption of keratinocytes adhesion and cleavage within the stratum granulosum which leads to bulla formation.The diagnosis is mainly clinical,based on the findings of tender erythroderma,bullae,and desquamation with a scalded appearance especially in friction zones,periorificial scabs/crusting,positive Nikolsky sign,and absence of mucosal involvement.Prompt empiric treatment with intravenous anti-staphylococcal antibiotic such as nafcillin,oxacillin,or flucloxacillin is essential until cultures are available to guide therapy.Clarithromycin or cefuroxime may be used should the patient have penicillin allergy.If the patient is not improving,critically ill,or in com-munities where the prevalence of methicillin-resistant S.aureus is high,vancomycin should be used.Conclusion A high index of suspicion is essential for an accurate diagnosis to be made and treatment promptly initiated.

Can baseline ML Flow test results predict leprosy reactions? An investigation in a cohort of patients enrolled in the uniform multidrug therapy clinical trial for leprosy patients in Brazil

Background:The predictive value of the serology to detection of IgM against the Mycobacterium leprae-derived phenolic glycolipid-I/PGL-I to identify leprosy patients who are at higher risk of developing reactions remains controversial.Whether baseline results of the ML Flow test can predict leprosy reactions was investigated among a cohort of patients enrolled in The Clinical Trial for Uniform Multidrug Therapy for Leprosy Patients in Brazil(U-MDT/CT-BR).Methods:This was a descriptive study focusing on the main clinical manifestations of leprosy patients enrolled in the U-MDT/CT-BR from March 2007 to February 2012 at two Brazilian leprosy reference centers.For research purposes,753 leprosy patients were categorized according to a modified Ridley-Jopling(R&J)classification and according to the development of leprosy reactions(reversal reaction/RR and erythema nodosum leprosum/ENL),and whether they had a positive or negative bacillary index/BI.Results:More than half of the patients(55.5%)reported leprosy reaction:18.3%(138/753)had a RR and 5.4%(41/753)had ENL.Leprosy reactions were more frequent in the first year following diagnosis,as seen in 27%(205/753)of patients,while 19%(142/753)developed reactions during subsequent follow-up.Similar frequencies of leprosy reactions and other clinical manifestations were observed in paucibacillary(PB)and multibacillary(MB)leprosy patients treated with U-MDT and regular MDT(R-MDT)(P=0.43 and P=0.61,respectively).Compared with PB patients,leprosy reactions were significantly more frequent in MB patients with a high BI,and more patients developed RR than ENL.However,RR and neuritis were also reported in patients with a negative BI.At baseline,the highest rate of ML Flow positivity was observed in patients with a positive BI,especially those who developed ENL,followed by patients who had neuritis and RR.Among reaction-free patients,81.9%were ML Flow positive,however,the differences were not statistically significant compared to reactional patients(P=0.45).Conclusions:MB and PB patients treated with R-MDT and U-MDT showed similar frequencies of RR and other clinical manifestations.Positive ML Flow tests were associated with MB leprosy and BI positivity.However,ML Flow test results at baseline showed limited sensitivity and specificity for predicting the development of leprosy reactions.